18 三体综合征儿童肝母细胞瘤的手术治疗:单中心研究。

IF 1.5 3区 医学 Q2 PEDIATRICS Pediatric Surgery International Pub Date : 2024-08-14 DOI:10.1007/s00383-024-05813-x
Kazuki Hirohara, Hirofumi Tomita, Naoki Shimojima, Ayano Tsukizaki, Teizaburo Mori, Hidehiro Minegishi, Atsushi Makimoto, Yuki Yuza, Kentaro Matsuoka, Akihiro Shimotakahara
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引用次数: 0

摘要

目的:近来,18 三体综合征患儿在恶性肿瘤方面得到了更积极的治疗。我们在此报告了 7 例完全切除的病例,并讨论了 18 三体综合征患者肝母细胞瘤的多学科治疗:方法:回顾2010年至2023年期间在研究中心接受治疗的18三体综合征患儿的病历:结果:69 例患者中有 6 例发展为肝母细胞瘤,其中 3 例接受了多学科治疗。此外,6 名患者由其他医院转诊,其中 4 人接受了多学科治疗。在接受多学科治疗的 7 名患者中,分别有 3 人、2 人和 2 人被归入治疗前病变程度(PRETEXT)分类 I 组、II 组和 III 组。三例患者接受了新辅助化疗,肿瘤缩小。所有病例均实现了完全切除,病理边缘安全。围手术期并发症包括1例循环衰竭和2例胆汁渗漏。四例患者接受了辅助化疗。术后观察期从 3 个月到 11 年不等,所有患者均无复发:结论:18 三体综合征并发肝母细胞瘤的儿童,如果心肺状况稳定,可能是化疗和手术的良好人选。
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Surgery for hepatoblastoma in children with trisomy 18: a monocentric study.

Purpose: Recently, children with trisomy 18 have been receiving more active treatment for malignancies. We report herein seven cases complete resection was achieved, and discuss multidisciplinary treatment for hepatoblastoma in patients with trisomy 18.

Method: The medical records of children with trisomy 18 who were treated at the study center between 2010 and 2023 were reviewed.

Result: Six of 69 patients had hepatoblastoma development, and three of these underwent multidisciplinary treatment. In addition, 6 patients had been referred by another hospital for treatment, and four of these underwent multidisciplinary treatment. Among the seven patients who underwent multidisciplinary treatment, three, two, and two were categorized in Pre-treatment Extent of Disease (PRETEXT) classification group I, II, and III, respectively. Neoadjuvant chemotherapy resulting in tumor reduction was performed in three cases. In all the cases, complete resection was achieved with pathologically safe margins. Perioperative complications included circulatory failure in one case and bile leakage in two cases. Adjuvant chemotherapy was administered in four cases. The postoperative observation period ranged from 3 months to 11 years, and all the patients are recurrence-free.

Conclusion: Children with trisomy 18 complicated with hepatoblastoma whose cardiopulmonary conditions are stable may be good candidates for chemotherapy and surgery.

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来源期刊
CiteScore
3.00
自引率
5.60%
发文量
215
审稿时长
3-6 weeks
期刊介绍: Pediatric Surgery International is a journal devoted to the publication of new and important information from the entire spectrum of pediatric surgery. The major purpose of the journal is to promote postgraduate training and further education in the surgery of infants and children. The contents will include articles in clinical and experimental surgery, as well as related fields. One section of each issue is devoted to a special topic, with invited contributions from recognized authorities. Other sections will include: -Review articles- Original articles- Technical innovations- Letters to the editor
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